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  • Title: Metastasizing chordoma in early childhood: a pathological and immunohistochemical study with review of the literature.
    Author: Sibley RK, Day DL, Dehner LP, Trueworthy RC.
    Journal: Pediatr Pathol; 1987; 7(3):287-301. PubMed ID: 3684809.
    Abstract:
    A 2 1/2-year-old female with a sphenooccipital-vertebral chordoma presented with neck pain, torticollis, fever, a lytic lesion of C2 vertebra, and bilateral nodular infiltrates in the lung. The lung biopsy revealed multiple tumor emboli by an enigmatic epithelioid-appearing neoplasm with immunohistochemical staining for vimentin, cytokeratin, and epithelial membrane antigen. A thorough roentgenographic evaluation disclosed a destructive, prepontine mass in the region of the clivus, erosion of the odontoid process, and compression of the cervical spinal cord. The patient died after a clinical course of 3 months. We identified 16 additional cases of metastasizing chordomas in the pediatric-age population; this case is the first to our knowledge with pathologically documented pulmonary metastasis at presentation.
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